Happy New Year.
Thank you for your touching emails and postings. Nicholas is doing well. He has had a few more seizures in the past few weeks and we have had to add a 12:30PM dose to his daily medications. I will be adding a tab about Nicholas past year of actual seizure, type of medication he was on at the time and the side effects Nicholas experienced. Hopefully it will be helpful to other families with kids that have Batten disease to see our experiences and where we are now. Currently Nicholas is still on Ativan and not completely seizure controlled but most importantly he has his old personality and spunk back. My next plan of action in the next few weeks is to investigate the Ketogenic Diet. I have a lot of resources for this diet but it was not my specialty when I worked as a dietitian.
This is a diet that is about 50% effective with controlling epilepsy. It is non-invasive plan but there are a few side effects one which is weight loss. Nicholas did loose about 6-7 pounds after he was in the hospital last fall. His baseline weight has always been at the high end of his ideal body weight. He had lost his desire to eat and feed himself and so I have been feeding him since September most meals. He would go all day not ask for food and not feed himself when food was in front of him. But in the past week I have noticed that he has been willing to sit down and feed himself at least half a meal. He uses his hands because it is difficult for him to see the food and maneuver the utensils. So I am glad he is getting his indepences back to want to feed himself but I give him a lot of encouragement. I’ll have a tab about this diet and our experiences if we choose to follow through with it. First I plan to contact a specialist at Children’s hospital in Boston about this ketogenic diet.
Nicholas is back in school after all the snow days, holidays and vacation. This transition back to school and leaving me in the morning has been difficult. His anxiety overrules him and he cries, screams and yells as I leave him with his LPN on the bus. She assures me once they get to school he has settled down and gets to business. This behavior is not new for Nicholas but it breaks my heart every time I leave him. I continue to work with his team of teachers at school to help his anxiety and keep to a routine.
We are still awaiting the final results of Nicholas’s CLN tests. We do know he does not have CLN3 and according to our Batten disease specialist she reported to us CLN5 testing is just about complete and does not identify a mutation. She also reported she is moving on to CLN6 and will also do deletional analysis.
On a good note. We have been enjoying all this snow and go sledding almost every day.
